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Chapter 12 - Autoimmune Causes of Medication-Resistant Epilepsy

Published online by Cambridge University Press:  20 August 2020

John M. Stern
Affiliation:
Geffen School of Medicine at UCLA, Los Angeles, CA
Raman Sankar
Affiliation:
Geffen School of Medicine at UCLA, Los Angeles, CA
Michael Sperling
Affiliation:
Jefferson Hospital for Neurosciences, Philadelphia, PA
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Summary

Epilepsy affects approximately 65 million people worldwide. Despite expanding treatment options, up to one-third of patients remain medically intractable[1]. With the exception of surgery in a subset of these patients, epilepsy treatment is usually limited to suppression of seizures with anti-seizure medications (ASMs). Accumulating data support an autoimmune aetiology in some patients [2,3]. Identification of neural antibodies allows a direct means of establishing an autoimmune cause in many of these cases[3–6]. Table 12.1 shows the best-known currently identified autoimmune epilepsy syndromes.

Type
Chapter
Information
Medication-Resistant Epilepsy
Diagnosis and Treatment
, pp. 100 - 117
Publisher: Cambridge University Press
Print publication year: 2020

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References

Kwan, P, Brodie, MJ. Early identification of refractory epilepsy. N Engl J Med 2000;342:314319Google Scholar
Greco, A, Rizzo, MI, De Virgilio, A, et al. Autoimmun Rev 2016;15:221225Google Scholar
Irani, SR, Bien, CG, Lang, B. Autoimmune epilepsies. Curr Opin Neurol 2011;24:146153CrossRefGoogle ScholarPubMed
Suleiman, J, Dale, RC. The recognition and treatment of autoimmune epilepsy in children. Dev Med Child Neurol 2015;57: 431440CrossRefGoogle ScholarPubMed
Graus, F, Titulaer, MJ, Balu, R, et al. Clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol 2016;15:391404Google Scholar
Brenner, T, Sills, GJ, Hart, Y, et al. Prevalence of neurologic autoantibodies in cohorts of patients with new and established epilepsy. Epilepsia 2013;54:10281035Google Scholar
De Vries, EE, van den Munckhof, B, Braun, KP, et al. Inflammatory mediators in human epilepsy: a systematic review and meta-analysis. Neurosci Biobehav Rev 2016;63:177190Google Scholar
Johnson, MR, Behmoaras, J, Bottolo, L, et al. Systems genetics identifies Sestrin 3 as a regulator of a proconvulsant gene network in human epileptic hippocampus. Nat Commun 2015;6:6031Google Scholar
Bien, CG, Vincent, A, Barnett, MH, et al. Immunopathology of autoantibody-associated encephalitides: clues for pathogenesis. Brain 2012;135:16221638CrossRefGoogle ScholarPubMed
Byun, JI, Lee, ST, Jung, KH, et al. Effect of immunotherapy on seizure outcome in patients with autoimmune encephalitis: a prospective observational registry study. PLoS One 2016;11:e0146455Google Scholar
Bello-Espinosa, LE, Rajapakse, T, Rho, JM, Buchhalter, J. Efficacy of intravenous immunoglobulin in a cohort of children with drug-resistant epilepsy. Pediatr Neurol 2015;52:509516Google Scholar
Toledano, M, Britton, JW, McKeon, A, et al. Utility of an immunotherapy trial in evaluating patients with presumed autoimmune epilepsy. Neurology 2014;82:15781586Google Scholar
Quek, AM, Britton, JW, McKeon, A, et al. Autoimmune epilepsy: clinical characteristics and response to immunotherapy. Arch Neurol 2012;69:582593Google Scholar
Ong, M, Kohane, IS, Cai, T, et al. Population-level evidence for an autoimmune etiology of epilepsy. J Am Med Assoc Neurol 2014;71:569574Google Scholar
Devinsky, O, Schein, A, Najjar, S. Epilepsy associated with systemic autoimmune disorders. Epilepsy Curr 2013;13:6268Google Scholar
Flanagan, EP, Kotsenas, AL, Britton, JW, et al. Basal ganglia T1 hyperintensity in LGI1-autoantibody faciobrachial dystonic seizures. Neurol Neuroimmunol Neuroinflamm 2015;2:e161CrossRefGoogle ScholarPubMed
Lai, M, Huijbers, MGM, Lancaster, E, et al. Investigation of LGI1 as the antigen in limbic encephalitis previously attributed to potassium channels: a case series. Lancet Neurol 2010;9:776785Google Scholar
Irani, SR, Michell, AW, Lang, B, et al. Faciobrachial dystonic seizures precede Lgi1 antibody limbic encephalitis. Ann Neurol 2011;69:892900Google Scholar
Irani, SR, Gelfand, JM, Bettcher, BM, et al. Effect of rituximab in patients with leucine rich, glioma-inactivated antibody-associated encephalopathy. J Am Med Assoc Neurol 2014;71:896900Google ScholarPubMed
Krogias, C, Hoepner, R, Müller, A, et al. Successful treatment of anti-Caspr2 syndrome by interleukin 6 receptor blockade through tocilizumab. J Am Med Assoc Neurol 2013;70:10561059Google Scholar
Peery, HE, Day, GS, Dunn, S, et al. Anti-NMDA receptor encephalitis: the disorder, the diagnosis and the immunobiology. Autoimmun Rev 2012;11:863872Google Scholar
Titulaer, MJ, McCracken, L, Gabilondo, I, et al. Treatment and prognostic factors for long-term outcome in patients with anti-NMDA receptor encephalitis: an observational cohort study. Lancet Neurol 2013;12:157165Google Scholar
Prud’homme, GJ, Glinka, Y, Wang, Q. Immunological GABAergic interactions and therapeutic applications in autoimmune diseases. Autoimmun Rev 2015;14:10481056Google Scholar
Petit-Pedrol, M, Armangue, T, Peng, X, et al. Encephalitis with refractory seizures, status epilepticus, and antibodies to the GABAA receptor: a case series, characterization of the antigen, and analysis of the effects of antibodies. Lancet Neurol 2014;13:276286Google Scholar
Lancaster, E, Lai, M, Peng, X, et al. Antibodies to the GABA(B) receptor in limbic encephalitis with seizures case series and characterization of the antigen. Lancet Neurol 2010;9:6776Google Scholar
Lai, M, Hughes, EG, Peng, X, et al. AMPA receptor antibodies in limbic encephalitis alter synaptic receptor location. Ann Neurol 2009;65:424434Google Scholar
Carvajal-González, A, Leite, MI, Waters, P, et al. Glycine receptor antibodies in PERM and related syndromes: characteristics, clinical features and outcomes. Brain 2014;137:21782192Google Scholar
Lancaster, E, Martinez-Hernandez, E, Titulaer, MJ, et al. Antibodies to metabotropic glutamate receptor 5 in the Ophelia syndrome. Neurology 2011;77:16981701CrossRefGoogle ScholarPubMed
Malter, MP, Helmstaedter, C, Urbach, H, et al. Antibodies to glutamic acid decarboxylase define a form of limbic encephalitis. Ann Neurol 2010;67:470478Google Scholar
Pradeep, K, Sinha, S, Mahadevan, A, et al. Clinical, electrophysiological, imaging, pathological and therapeutic observations among 18 patients with Rasmussen’s encephalitis. J Clin Neurosci 2016;25:96104CrossRefGoogle ScholarPubMed
Marras, CE, Granata, T, Franzini, A, et al. Hemispherectomy and functional hemispherectomy: indications and outcome. Epilepsy Res 2010;89:104112Google Scholar
Hanly, JG, Urowitz, MB, Su, L, et al. Seizure disorders in systemic lupus erythematosus results from an international, prospective, inception cohort study. Ann Rheum 2012;71:15021509CrossRefGoogle ScholarPubMed
Olmez, I, Moses, H, Sriram, S, et al. Diagnostic and therapeutic aspects of Hashimoto’s encephalopathy. J Neurol Sci 2013;331: 6771CrossRefGoogle ScholarPubMed
Howell, KB, Katanyuwong, K, Mackay, MT, et al. Long-term follow-up of febrile infection-related epilepsy syndrome. Epilepsia 2012;53:101110CrossRefGoogle ScholarPubMed
Gaspard, N, Foreman, BP, Alvarez, V, et al. Critical Care EEG Monitoring Research Consortium (CCEMRC). New-onset refractory status epilepticus: etiology, clinical features, and outcome. Neurology 2015;85:16041613Google Scholar
Najjar, S, Pearlman, D, Zagzag, D, Devinsky, O. Spontaneously resolving seronegative autoimmune limbic encephalitis. Cogn Behav Neurol 2011;24:99105Google Scholar
Beghi, E, Shorvon, S. Antiepileptic drugs and the immune system. Epilepsia 2011;52(Suppl 3):4044Google Scholar
Dinarello, CA, Simon, A, van der Meer, JW. Treating inflammation by blocking interleukin-1 in a broad spectrum of diseases. Nat Rev Drug Discov 2012;11:633652Google Scholar

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